1/8. Sclerosing mesenteritis seen clinically as pancreatic pseudotumor: two cases and a review.Sclerosing mesenteritis is an uncommon nonneoplastic inflammatory process in the mesentery that is seen as a pseudotumor, usually involving the small bowel mesentery, the mesenteric fat, and less commonly, the mesentery of the large bowel. We report two cases of sclerosing mesenteritis and review the literature on this rare disease. Both patients had pain, profound weight loss, and a mass on computed tomography (CT) scan of the abdomen. The provisional diagnosis was pancreatic neoplasm on the basis of clinical presentation and imaging studies. The diagnosis of sclerosing mesenteritis was established by histologic findings in biopsy material obtained at laparotomy in both cases. Interval histologic studies in one patient who had a high CA 19-9 level, progressive biliary ductal and partial duodenal compression, revealed a transitional histologic pattern from predominant inflammation and fat necrosis to predominant fibrosis. This may explain the varied descriptive terms used in the literature to describe this entity.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/8. Colonic carcinoma: a case report in a child and review literature.This article reports one case of child colonic carcinoma. This is a rare disease in children which usually occurs in predisposing conditions, e.g. ulcerative colitis, familial polyposis coli, Gardner's syndrome, Turcot's syndrome and Peutz-Jegher's syndrome. The patient in this report was 12 years old. He presented with chronic intermittent colicky abdominal pain and uncorrectable iron deficiency anemia for 7 months prior to definite diagnosis. This report also reviews the literature about colorectal carcinoma in children. physicians can make an early diagnosis with a high index of suspicion if they cannot explain clearly what causes abdominal pain. Further investigations should be performed, thereby, avoiding delayed diagnosis and improving survival rate.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/8. Mesenteric panniculitis of the colon with obstruction of the inferior mesenteric vein: report of a case.Mesenteric panniculitis is a rare disease characterized by nonspecific inflammation of the fat tissue of the mesentery. We present an extremely rare case of mesenteric panniculitis of the sigmoid colon, complicated by occlusion of the inferior mesenteric vein. A 75-year-old male presented with a one-month history of abdominal distention and abdominal mass without pain. physical examination revealed a firm mass in the lower abdomen. barium enema study demonstrated rugged mucosa and a serrated contour in the rectosigmoid colon. Computed tomography showed that the mass arose from the mesentery, which surrounded the mesenteric vessels. The density of the mass was slightly higher than that of fatty tissue. Based on these radiologic findings, the patient was diagnosed as having mesenteric panniculitis of the rectosigmoid colon. colonoscopy showed narrowing with edematous mucosa in the rectosigmoid colon, whereas marked dilated vessels were noted in the proximal portion of the sigmoid colon. angiography showed occlusion of the inferior mesenteric vein, with venous flow returning via a collateral vein. The patient was observed without medication because his condition was satisfactory. His symptoms subsequently disappeared during a period of several weeks. The mass in the lower abdomen gradually diminished in size, disappearing three months later. Computed tomography and barium enema showed improvement of the lesion. The favorable outcome of the present case was probably because of formation of a collateral vein. The present case suggests that aggressive therapy for mesenteric panniculitis should be avoided, because the outcome of this disorder is good, even when there is obstruction of vessels.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/8. abdominal pain in children: a case of acalculous cholecystitis.acalculous cholecystitis is a rare disease in children. Most cases are associated with systemic infections or with autoimmune pathologies, but it may also occur without predisposing factors. A case observed in an otherwise healthy child is reported.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/8. Cystic dystrophy of the duodenal wall in a heterotopic pancreas.Cystic dystrophy of the duodenal wall in heterotopic pancreas is a rare disease affecting younger men in particular. On account of its difficult diagnosis and nonspecific signs, it was first described in 1970. A 51-year-old male patient was admitted for clarification of several months of severe pain in the upper abdomen accompanied by weight-loss, and due to ultrasonically established suspicion of carcinoma in the head of the pancreas. Clinical examination and laboratory tests were nonsignificant. barium meal and esophagogastroduodenoscopy revealed a severe deformation of the duodenum. biopsy of the duodenal mucosa detected moderate inflammatory changes. Ultrasound examination showed cystic changes in the duodenal wall and in the vicinity of the head of the pancreas. Only with the aid of endoscopic ultrasound, could bizarre cystic changes in the submucosa of the duodenal wall be detected, which was also confirmed by computer tomography. The signs of disease are nonspecific. Duodenal biopsy also does not reveal its cause since the typical lesions lie deeper in the submucosa or muscularis propria. Cystic changes with fibrosis, pancreatic ducts and lobi without anatomic or vascular connection to the pancreas are found. The anomaly involves, above all, the stomach and duodenum. Prior to the introduction of imaging techniques such as endoscopic ultrasonography, computer tomography and nuclear magnetic resonance, the diagnosis could only be made by means of an operative procedure. Treatment is primarily surgical; smaller cysts can be treated by endoscopic fenestration. Pharmacological treatment with somatostatin is rarely performed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/8. Primary pancreatic lymphoma presenting as acute pancreatitis.Primary pancreatic lymphoma is a rare disease and usually presents with nonspecific symptoms like abdominal pain, weight loss, or jaundice. Here we report a case of primary pancreatic lymphoma presenting as acute pancreatitis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/8. Recurrent leiomyomatosis peritonealis disseminata after hysterectomy and bilateral salpingo-oophorectomy during combined hormone replacement therapy.Leiomyomatosis peritonealis disseminata is a rare disease characterised by the occurrence of multiple smooth muscle cells building up tumour nodules within the peritoneal cavity. Approximately 50 cases of this disease have been reported in the world literature. To our knowledge, this is the first case reporting the recurrence of leiomyomatosis peritonealis disseminata during combined hormone replacement therapy after hysterectomy and bilateral salpingo-oophorectomy and six laparotomies because of recurrence of the disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/8. Lymphangiomyomatosis arising in the pelvic cavity: a case report.lymphangioleiomyomatosis (LAM) is a rare disease usually occurring in young women of child-bearing age. It is characterized by a distinctive proliferation of lymphatic smooth muscle cells, especially occurring in the pulmonary parenchyme. The majority of primary LAM occurs in the lung, but there are a few reports of extrapulmonary cases. We report a case of a 21-yr-old female who first complained of low abdominal pain and was referred from a local clinic with the impression of an ovarian cyst. Gynecologic ultrasonography revealed a large posterior pelvic mass with an irregular echogenicity measuring 9.7 x 4.2 cm in size. Pelviscopy showed a large, thin walled, partly cystic, pelvic mass. The mass was partly removed. Microscopically, the mass was characterized by a haphazard proliferation of smooth muscle cells arranged in fascicular, trabecular, and papillary patterns around a ramifying network of endothelium-lined spaces. The cells were plump or epithelioid with abundant eosinophilic cytoplasm and showed a positive reaction for both alpha-smooth muscle actin and HMB-45 antigen. Surgical and pathological findings were consistent with pelvic retroperitoneal LAM. Despite the numerous treatment attempts, the patient suffered from intractable chylous ascites and developed pulmonary LAM and died due to severe respiratory distress.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |